%0 Journal Article
%@ 1742-2094
%A Jarius, Sven
%A Ruprecht, Klemens
%A Kleiter, Ingo
%A Borisow, Nadja
%A Asgari, Nasrin
%A Pitarokoili, Kalliopi
%A Pache, Florence
%A Stich, Oliver
%A Beume, Lena-Alexandra
%A Hümmert, Martin W.
%A Trebst, Corinna
%A Ringelstein, Marius
%A Aktas, Orhan
%A Winkelmann, Alexander
%A Buttmann, Mathias
%A Schwarz, Alexander
%A Zimmermann, Hanna
%A Brandt, Alexander U.
%A Franciotta, Diego
%A Capobianco, Marco
%A Kuchling, Joseph
%A Haas, Jürgen
%A Korporal-Kuhnke, Mirjam
%A Lillevang, Soeren Thue
%A Fechner, Kai
%A Schanda, Kathrin
%A Paul, Friedemann
%A Wildemann, Brigitte
%A Reindl, Markus
%C London
%D 2016
%F heidok:22059
%I BioMed Central
%J Journal of Neuroinflammation
%N 279
%P 1-16
%T MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 1: Frequency, syndrome specificity, influence of disease activity, long-term course, association with AQP4-IgG, and origin
%U https://archiv.ub.uni-heidelberg.de/volltextserver/22059/
%V 13
%X Background: Antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) have been suggested to play a role in a subset of patients with neuromyelitis optica and related disorders.   Objective: To assess (i) the frequency of MOG-IgG in a large and predominantly Caucasian cohort of patients with optic neuritis (ON) and/or myelitis; (ii) the frequency of MOG-IgG among AQP4-IgG-positive patients and vice versa; (iii) the origin and frequency of MOG-IgG in the cerebrospinal fluid (CSF); (iv) the presence of MOG-IgG at disease onset; and (v) the influence of disease activity and treatment status on MOG-IgG titers.   Methods: 614 serum samples from patients with ON and/or myelitis and from controls, including 92 follow-up samples from 55 subjects, and 18 CSF samples were tested for MOG-IgG using a live cell-based assay (CBA) employing full-length human MOG-transfected HEK293A cells.   Results: MOG-IgG was detected in 95 sera from 50 patients with ON and/or myelitis, including 22/54 (40.7%) patients with a history of both ON and myelitis, 22/103 (21.4%) with a history of ON but no myelitis and 6/45 (13.3%) with a history of longitudinally extensive transverse myelitis but no ON, and in 1 control patient with encephalitis and a connective tissue disorder, all of whom were negative for AQP4-IgG. MOG-IgG was absent in 221 further controls, including 83 patients with AQP4-IgG-seropositive neuromyelitis optica spectrum disorders and 85 with multiple sclerosis (MS). MOG-IgG was found in 12/18 (67%) CSF samples from MOG-IgG-seropositive patients; the MOG-IgG-specific antibody index was negative in all cases, indicating a predominantly peripheral origin of CSF MOG-IgG. Serum and CSF MOG-IgG belonged to the complement-activating IgG1 subclass. MOG-IgG was present already at disease onset. The antibodies remained detectable in 40/45 (89%) follow-up samples obtained over a median period of 16.5 months (range 0–123). Serum titers were higher during attacks than during remission (p < 0.0001), highest during attacks of simultaneous myelitis and ON, lowest during acute isolated ON, and declined following treatment.   Conclusions: To date, this is the largest cohort studied for IgG to human full-length MOG by means of an up-to-date CBA. MOG-IgG is present in a substantial subset of patients with ON and/or myelitis, but not in classical MS. Co-existence of MOG-IgG and AQP4-IgG is highly uncommon. CSF MOG-IgG is of extrathecal origin. Serum MOG-IgG is present already at disease onset and remains detectable in the long-term course. Serum titers depend on disease activity and treatment status.